The outcome of the vein of Galen aneurysmal malformation cases diagnosed prenatally.

Vein of Galen aneurysmal malformation (VGAM) is a rare foetal anomaly associated with neurodevelopment delay, cardiac failure, and even perinatal death. We aimed to assess prenatal features of VGAM and describe postnatal outcomes. This was a retrospective study involving six foetuses diagnosed with VGAM prenatally in two centres. All of the cases underwent foetal neurosonography and echocardiography. The presence of ventriculomegaly, intracranial haemorrhage and cardiac failure was recorded. Pregnancy and neonatal outcome information were obtained from medical records. The mean gestational age at diagnosis was 31.1 ± 5.1 weeks, and the mean size of VGAM was 29.2 ± 5.2 × 26.4 ± 3.3 mm. Ventriculomegaly was detected in five of six (83.3%) cases. Intracranial haemorrhage was present in five (83.3%) cases. Cardiac failure was shown in four (66.6%) foetuses. Three foetuses underwent termination of pregnancy (TOP); in two cases, neonatal death occurred. One patient was treated with endovascular embolisation, and there was no cardiac problem or neurodevelopment delay. Prenatally diagnosed VGAM have a poor prognosis, mainly if a cardiac failure or neurological consequences (intracranial haemorrhage, hydrocephaly) are present in utero.Impact StatementWhat is already known on this subject? VGAM is the most common cerebral arteriovenous malformation detected prenatally, and it can lead to severe consequences in the perinatal period.What do the results of this study add? The accuracy of foetal neurosonography is excellent for detecting VGAM and associated brain abnormalities. Foetal echocardiography is mandatory for the prediction of prognosisWhat are the implications of these findings for clinical practice and/or further research? VGAM is associated with severe brain injury, cardiac failure, and the prognosis is generally poor. We need predictors to identify those expected to benefit from postnatal therapy.

Temporal bone hemangioendothelioma as a rare vascular tumor in childhood: case report and review of the literature.

BACKGROUND: Hemangiondothelioma is a rare vascular tumor that can occur in the bone. Temporal bone involvement has been reported extremely rare in the literature. CASE: Radiological examination of a one-year-old girl who was admitted due to facial paralysis revealed vascular tumor of the temporal bone and Galen vein aneurysm. Pathological examination showed retiform hemangioendothelioma. She was treated with propranolol, prednisolone, vincristine, and endovascular embolization followed by oral sirolimus. With sirolimus treatment, a partial response was obtained first, then the tumor remained stable and sirolimus treatment was discontinued. No progression was observed in the disease after discontinuation of treatment. CONCLUSION: In this article, a case of hemangioendothelioma originating from the temporal bone is discussed in the light of other case reports in the literature.

Prenatal Diagnosis of Galen Vein Aneurysm Using Ultrasonography and Magnetic Resonance Imaging and Perinatal and LongTerm Neurological Outcomes: A Case Series / Diagnóstico do aneurisma de veia de Galeno por meio de ultrassonografia e ressonância magnética e resultados perinatais e neurológicos: série de casos

Abstract Objective To describe the prenatal diagnosis of Galen vein aneurysm (GVA) based on ultrasonography and magnetic resonance imaging (MRI) in a series of cases, as well as its postnatal outcomes and follow-up until 4 years of age. Methods A retrospective longitudinal study was performed, analyzing a database comprising seven cases of prenatal diagnosis of GVA at two Brazilian institutions from February of 2000 to May of 2012. The following data were evaluated: gestational age at diagnosis, GVA dimensions on ultrasonography, associated fetal changes, findings on fetal echocardiography, gestational age at delivery, type of delivery, birth weight, Apgar score at the 1st and 5th minutes, neonatal outcomes, and survival with follow-up until 4 years of age. Results The mean gestational age ± standard deviation on the prenatal diagnosis of GVA based on ultrasonography was 25±4.9 weeks. The mean length of GVA was 3.2±0.4 cm. The mean gestational age at birth was 37.5±0.7 weeks, and a cesarean section was performed in 85.7% of the cases (6/7). The mean birth weight was 3,070±240.4 g. The total survival rate was 42.8% (4/7), with three neonatal deaths. Of the four survivors, three presented with normal neuropsychomotor development until 4 years of age and only one showed serious neurological sequelae. Ultrasonography and MRI showed similar findings for all seven cases. Conclusions Galen Vein Aneurysm is associated with a high neonatal death rate. Therefore, its prenatal diagnosis is essential for parent counseling and follow-up at tertiary care institutions.

Resumo Objetivo Descrever o diagnóstico pré-natal de uma série de casos de aneurisma de veia de Galeno (AVG) por meio de ultrassonografia e ressonância magnética (RM), bem como os resultados pós-natais e acompanhamento até 4 anos de vida. Métodos Realizou-se um estudo retrospectivo longitudinal com análise de banco de dados de sete casos de diagnóstico pré-natal de AVG em dois serviços brasileiros entre fevereiro de 2000 e maio de 2012. Foram avaliados a idade gestacional ao diagnóstico, dimensões do AVG na ultrassonografia, alterações fetais associadas, achados da ecocardiografia fetal, idade gestacional ao parto, tipo de parto, peso ao nascimento, índice de Apgar no 1° e 5° minutos, resultados neonatais, e sobrevida com acompanhamento até 4 anos de idade. Perinatais Resultados A idade gestacional média±desvio-padrão ao diagnóstico pré-natal do AVG pela ultrassonografia foi de 25±4,9 semanas. O comprimento médio do AVG foi 3,2±0,4 cm. A idade gestacional média ao nascimento foi 37,5±0,7 semanas, sendo que, em 85,7% dos casos (6/7) o parto foi cesáreo. O peso médio ao nascimento foi de 3.070±240,4 gramas. A sobrevida total foi de 42,8% (4/7), com três óbitos neonatais. Dos quatro sobreviventes, três apresentaram desenvolvimento neuropsicomotor normal até a idade de 4 anos, sendo que apenas um apresentou sequelas neurológicas graves. Ultrassonografia e RM apresentaram achados semelhantes nos sete casos. Conclusões O AVG está associado à elevada taxa de óbito neonatal, sendo, portanto, fundamental o seu diagnóstico pré-natal precoce para aconselhamento dos pais e seguimento em serviço terciário.

Role of 3D power Doppler sonography in early prenatal diagnosis of Galen vein aneurysm.

Vein of Galen aneurysm malformation (VGAM) is a rare congenital vascular anomaly. Although the cause of VGAM remains to be elucidated, the current hypothesis is persistence of the embryonic vascular supply, which leads to progressive enlargement and formation of the aneurysmal component of a typical VGAM. Here, we present a 36-year-old woman at 23 weeks' gestation (gravida 3, para 2) who was evaluated using 3D power Doppler sonography for the prenatal diagnosis of a vein of Galen aneurysm. Investigation using 3D power Doppler sonography allowed for a non-invasive yet diffuse and detailed prenatal assessment of VGAM. Thus, we suggest that prenatal sonography with 3D power Doppler may be an option in cases of VGAM.

Aneurisma de la vena de Galeno / Galen's vein aneurysm

La malformación arteriovenosa de la vena de Galeno es una enfermedad congénita poco frecuente que se produce a partir de un defecto en la fusión de las venas cerebrales internas y que, debido a la baja resistencia, produce un cuadro de falla cardíaca de alto gasto. Las manifestaciones clínicas son muy variables y dependen de la edad de presentación. El pronóstico depende del tamaño del aneurisma. Se presenta el caso de un recién nacido del sexo masculino, con diagnóstico prenatal de aneurisma de la vena de Galeno y, desde el nacimiento, graves signos de insuficiencia cardíaca congestiva de difícil tratamiento. Se realiza una revisión sobre el tema

Arteriovenous malformation of Galen's vein is an uncommon congenital disease present from a defect in internal cerebral veins fusion and that due to the low resistance, produces a high output heart failure. Clinical manifestations are very variables depending on age of presentation. Prognosis depends on the size of aneurysm. This is the case of a male newborn diagnosed prenatally with Galen's vein aneurysm and from his birth severe signs of congestive heart failure of difficult treatment. A literature review on this subject is made